血管性血友病因子抗原检测在血友病A与血管性血友病鉴别诊断中的应用

目的评估血管性血友病因子(vWF)抗原检测在血友病(vWD)患者和轻中度血友病A患者鉴别诊断中的价值。方法从2010年8月到2015年12月在我中心检测凝血因子Ⅷ活性低于45%,而且根据临床表现及家族史怀疑为血管性血友病的患者77例,用酶联免疫法和免疫比浊法检测vWF抗原。结果检测的77例患者中29例患者VWF抗原水平低于35%,为vWD患者;48例未检出vWF抗原水平降低。其中女性患者16例中11例vWF抗原水平降低(68.75%),诊断为血管性血友病患者的几率要高于男性患者(29.51%),差异有统计学意义(P0.01)结论凝血因子Ⅷ活性低的患者,特别是女性患者,需要检测vWF抗原水平,筛查是否为vWD。vWF抗原水平检测能很好的满足临床需求,为vWD诊断提供依据,会得到越来越广泛的应用。

Application of von Willebrand Factor Antigen test in the Differential Diagnosis of von Willebrand Disease and Hemophilia A

Objective To evaluate the value of von Willebrand Factor (vWF)antigen assay in the differential diagnosis of von Willebrand Disease (vWD) and Hemophilia A.Methods From August 2010 to December 2015,we collected 77 patients with FⅧ:C ＜ 45％,which show the clinical symptom and family history of vWD.These patients were tested vWF antigen by enzyme-linked immunosorbent assay (ELISA) and immunoturbidimetry assay.Results Among the 77 patients,twenty-nine patients were diagnosed vWD with low VWF antigen(＜ 35％).Female patients had more probability been diagnosed vWD(68.75％)than male patients(29.51％),the difference had statistical significance (P ＜ 0.01).Conclusion Patients with low FⅧ:C,especially female patients,should consider vWD screening.vWF antigen assay is valuable in the diagnosis of vWD and more vWD will be diagnosed correctly in the future.